TY - JOUR
T1 - Synchronous Central Nervous System Atypical Teratoid/Rhabdoid Tumor and Malignant Rhabdoid Tumor of the Kidney
T2 - Case Report of a Long-Term Survivor and Review of the Literature
AU - Abu Arja, Mohammad H.
AU - Patel, Priyal
AU - Shah, Summit H.
AU - Auletta, Jeffery J.
AU - Meyer, Erin K.
AU - Conley, Suzanne E.
AU - Aldrink, Jennifer H.
AU - Pindrik, Jonathan A.
AU - AbdelBaki, Mohamed S.
PY - 2018/3
Y1 - 2018/3
N2 - Background Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) with synchronous or metachronous extra-CNS disease is a rare childhood malignancy with a dismal prognosis. Case Description We report a 7-week-old female with metastatic AT/RT and synchronous malignant rhabdoid tumor of the kidney who received an intensive multimodal approach combining surgical resection, intrathecal chemotherapy, and high-dose chemotherapy with autologous peripheral blood stem cell transplant (PBSCT). She is currently 24 months old without any evidence of disease. In addition, we completed an extensive literature review of cases with CNS AT/RT and synchronous or metachronous extra-CNS primary tumors. To date, 31 pediatric cases have been reported, and the median overall-survival was 6 months after diagnosis. The only 3 survivors received autologous PBSCT, and 2 of these patients had complete resection of their CNS tumor. Conclusions The rarity of CNS AT/RT with extra-CNS primary disease and the lack of standard treatment contribute to its reported dismal prognosis. We report a case of a long-term survivor with metastatic AT/RT and synchronous extra-CNS primary tumor. Maximal surgical resection, intrathecal chemotherapy, and consolidative autologous PBSCT may improve prognosis and avoid radiation.
AB - Background Atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) with synchronous or metachronous extra-CNS disease is a rare childhood malignancy with a dismal prognosis. Case Description We report a 7-week-old female with metastatic AT/RT and synchronous malignant rhabdoid tumor of the kidney who received an intensive multimodal approach combining surgical resection, intrathecal chemotherapy, and high-dose chemotherapy with autologous peripheral blood stem cell transplant (PBSCT). She is currently 24 months old without any evidence of disease. In addition, we completed an extensive literature review of cases with CNS AT/RT and synchronous or metachronous extra-CNS primary tumors. To date, 31 pediatric cases have been reported, and the median overall-survival was 6 months after diagnosis. The only 3 survivors received autologous PBSCT, and 2 of these patients had complete resection of their CNS tumor. Conclusions The rarity of CNS AT/RT with extra-CNS primary disease and the lack of standard treatment contribute to its reported dismal prognosis. We report a case of a long-term survivor with metastatic AT/RT and synchronous extra-CNS primary tumor. Maximal surgical resection, intrathecal chemotherapy, and consolidative autologous PBSCT may improve prognosis and avoid radiation.
KW - Atypical teratoid/rhabdoid tumor
KW - Autologous peripheral blood stem cell transplant
KW - Intrathecal chemotherapy
KW - SMARCB1
KW - Synchronous/metachronous rhabdoid tumors
UR - http://www.scopus.com/inward/record.url?scp=85039708435&partnerID=8YFLogxK
U2 - 10.1016/j.wneu.2017.11.158
DO - 10.1016/j.wneu.2017.11.158
M3 - Article
C2 - 29223518
AN - SCOPUS:85039708435
SN - 1878-8750
VL - 111
SP - 6
EP - 15
JO - World neurosurgery
JF - World neurosurgery
ER -